Ruptured aneurysms of sinus of Valsalva with bicuspid aortic valve--a case report of an aged patient (72 years old)

A 72-year-old man with ruptured aneurysms of the sinus of Valsalva and a bicuspid aortic valve was operated. Ruptured aneurysms were found in the noncoronary sinus region, and they were opened to the commissure between the anterior cusp and septal cusp of the tricuspid valve. The tissue around the aneurysm was very weak, so closure of the ruptured hole was carried out from the inside of both aorta and right atrium. Ruptured aneurysm of Valsalva is very rare in a aged patient. In this case, it is considered that the bicuspid aortic valve associated with malformations of the aortic cusp caused a weakness of the sinus wall, and this region gradually became aneurysmal.     

Congenital aortic regurgitation caused by a rudimentary noncoronary cusp: Report of a case

A 14-year-old asymptomatic boy was admitted to our department for investigation of a diastolic murmur which had been discovered by his family doctor during a routine examination. Echocardiography showed aortic regurgitation with dilatation of the left ventricle. Inspection of the aortic valve at the time of operation revealed normal left and right cusps with a rudimentary noncoronary cusp. An aortic commissuro-plication was performed and a new bicuspid aortic valve successfully reconstructed. His postoperative course was uneventful and he has been well and leading an active life since his discharge from hospital.     

Congenital aortic stenosis with hypoplasia of the left sinus of Valsalva. Anatomic reconstruction of the aortic root

Evaluation of the aortic root in 13 patients with congenital aortic stenosis aged 2.5 to 24 years (mean 8.3 years) has revealed morphologic characteristics of asymmetry of the aortic root caused by a small (hypoplastic) left sinus of Valsalva associated with a supravalvular ridge above the left coronary ostium and dysplasia of the aortic valve. The asymmetry resulted in folding and buckling of the left aortic cusp. The aortic valve was classified as bicuspid in 11 of the 13 patients. Preoperative aortography was characteristic and revealed the diagnosis in all patients. The average left ventricle-aorta systolic pressure gradient was 81 mm Hg. Operative repair consisted of an oblique aortotomy extended in a spiral fashion to the right and posteriorly into the left sinus of Valsalva. Seven patients had further mobilization of the posterior commissure with a second incision to the right of the commissure into the noncoronary sinus. Slightly fused valve commissures were opened in 12 patients. Aortic root reconstruction was accomplished with a spiral Dacron patch and posterior commissural repositioning. Follow-up catheterization at 9 to 35 months (mean 24 months) in five patients demonstrated an average outflow tract systolic gradient of 28 mm Hg and a more symmetrical appearance of the aortic root.     

Comparison of Three Different Surgical Methods in Aortic Root Aneurysms: Long-Term Results

Abstract Background: Degenerative ascending aortic aneurysms frequently present with aortic valve pathology. If only the noncoronary sinus of Valsalva is dilated, replacement of the noncoronary sinus by tailoring the supracoronary graft with or without aortic valve replacement (AVR) can be a simple operation strategy. We sought to describe our experiences in this kind of operation, and compare them with a relatively homogeneous group of patients who underwent the classical Bentall operation. Methods: Between January 1997 and June 2007, 99 patients who had dilated ascending aortas with root dilatation and aortic valve pathology underwent operation. Patients were divided into three groups according to the surgical procedure. Classical Bentall operation was applied in 54 patients with dilated sinuses (Group 1). The other patients with dilated noncoronary sinus underwent either ascending aortic replacement with noncoronary sinus replacement (NSR, n = 27) (Group 2), or separate AVR and ascending aortic replacement with NSR (AVR + NSR, n = 18) (Group 3). Results: There were significant reduction of aortic root in Group 2 (Z =?4.560, p < 0.001), and Group 3 (Z =?3.758, p < 0.001). Degree of aortic regurgitation was decreased from 1.56 ± 0.5 to 0.67 ± 0.5 postoperatively in Group 2 (Z =?3.874, p < 0.001). Hospital mortality was six (6.1%) (three in Group 1; three in Group 2). Late mortality rate was 6.1% (three in Group 1, three in Group 2). The type of operation was not found to be an independent predictor of overall mortality. No patients in the NSR and AVR‐NSR groups had aortic root aneurysm, and there were no reoperations or verified deaths caused by aortic root aneurysm in these patients. Conclusions: Noncoronary cusp replacement for aortic root remodeling in patients with ascending aortic aneurysm is a technically simple and durable operation. Short‐ and mid‐term results of this method were favorable compared to the Bentall procedure.     

Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation

BACKGROUND: Patients with bicuspid aortic valve malformations are at an increased risk of aortic dilatation, aneurysm formation, and dissection. Vascular tissues with deficient fibrillin-1 microfibrils release matrix metalloproteinases, enzymes that weaken the vessel wall by degrading elastic matrix components. In bicuspid aortic valve disease a deficiency of fibrillin-1 and increased matrix metalloproteinase matrix degradation might result in aortic degeneration and dilatation. METHODS: Samples of the pulmonary artery and aorta were obtained from surgical patients with bicuspid aortic valves (n = 21) and tricuspid aortic valves (n = 16). RESULTS: Fibrillin-1 content was reduced in bicuspid aortic valve aortas compared with that seen in tricuspid aortic valve aortas (P =.001), whereas the associated matrix components, elastin and collagen, were unchanged (P =.51 and P =.21). Reductions of aortic fibrillin-1 content were independent of valve function and patient age. Compared with tricuspid aortic valve aorta, matrix metalloproteinase 2 activity was increased more than 2-fold in bicuspid aortic valve aortas (P =.04) and correlated positively with aortic diameter (r = 0.74, P =.05). Matrix metalloproteinase 9 activity was not significantly different. Fibrillin-1 content was also reduced in the pulmonary arteries of patients with bicuspid aortic valves (P =.06), suggesting a systemic deficiency of fibrillin-1. Promatrix metalloproteinase 2 was increased (P =.04), reflecting an increased production of matrix metalloproteinase 2 in these fibrillin-1-deficient tissues, whereas active matrix metalloproteinase 2 and matrix metalloproteinase 9 species were unchanged, and correspondingly, the pulmonary arteries were not dilated. CONCLUSIONS: Deficient fibrillin-1 content in the vasculature of patients with bicuspid aortic valves might trigger matrix metalloproteinase production, leading to matrix disruption and dilatation. This process of vascular matrix remodeling in patients with bicuspid aortic valves offers novel therapeutic targets to prevent the aortic degeneration and dilatation characteristic of this disease.     

A case of ascending aortic dissection after aortic valve replacement in congenital bicuspid aortic valve]

Congenital bicuspid aortic valve is a risk factor of aortic dissection, but the case is rare in Japan. Several reports described ascending aortic aneurysm after aortic valve replacement. In these reports, most of aneurysms were false aneurysm, but the cases of ascending aortic dissection were rare. In this case, dissecting aneurysm of the ascending aorta occurred 4 years after aortic valve replacement, which was performed with mechanical prosthesis because of infective endocarditis, and it was repaired successfully by the modified Cabrol's method. This case was congenital bicuspid aortic valve, and had already been complicated with moderate aortic dilatation in the ascending aorta. In patients of congenital bicuspid aortic valve with aortic dilatation, consideration of complete replacement of the ascending aorta with aortic valve replacement is important.     

主动脉根部外科解剖及其与毗邻结构关系

目的：测量主动脉根部不同高度的口径大小，并观察主动脉窦与邻近结构的关系。方法：用30例正常成人甲醛固定心脏标本，测量主动脉根部不同高度的口径和主动脉瓣的大小，观察主动脉窦与邻近结构的关系。结果：（1）主动脉窦中部（Sinus)＞主动脉窦管结合部上1cm(STJ1)＞主动脉窦管结合处（STJ0）＞主动脉瓣环基底（Base）。经方差分析检验，P＜0．05，说明主动脉根部4个高度口径大小的差别具统计学意义。（2）主动脉瓣的瓣高、瓣附着缘长和瓣游离缘长的测量结果显示。（3）二尖瓣前瓣中轴线与主动脉窦的关系显示，二尖瓣前瓣中轴线86．6％位于左冠状动脉窦与无冠状动脉窦之间。（4）右心房主动脉隆凸与主动脉窦的关系显示，右心房主动脉隆凸由无冠状动脉窦形成者占73．3％，由无冠状动脉窦和右冠状动脉窦共同形成者占26．7％。（5）左、右肺动脉瓣交界点与主动脉窦的关系显示，左、右肺动脉瓣交界点对向左、右冠状动脉窦之间者占80％。结论：测量结果有助于心外科手术的开展。     

Bicuspid aortic valve disease and pulmonary autograft root dilatation after the Ross procedure: a clinicopathologic study.

OBJECTIVE: Bicuspid aortic valve disease has been associated with histologic abnormalities of the aortic root. Recent reports have suggested similar alterations may exist in the pulmonary artery of patients with bicuspid aortic valve. The present study was undertaken to define the histologic condition of the aortic and pulmonary artery root in bicuspid aortic valve disease and the relationship with pulmonary autograft root dilatation after the Ross procedure. METHODS: In 17 patients undergoing aortic root replacement with the pulmonary autograft, biopsy specimens of the aortic root and pulmonary artery trunk were collected. Clinical and histologic findings of patients with bicuspid aortic valves were compared with those with tricuspid aortic valves. RESULTS: There were 9 patients (8 male, 1 female) with bicuspid aortic valve (group 1) and 8 (all male) with tricuspid aortic valve (group 2). Mean age was comparable (24.4 +/- 9.8 vs 23.6 +/- 10.8 years, P =.9). Aortic insufficiency as an indication for operation was more common in group 1 (9/9 vs 5/8, P =.007), whereas preoperative aortic root dilatation was equally prevalent (4/9 vs 1/8, P =.1). Prior aortic valve repair had been performed in 2 patients (1/9 vs 1/8, P =.9). Prevalence of cystic medionecrosis of the aortic wall was similar in the 2 groups (4/9 vs 3/8, P =.6). Cystic medionecrosis of the pulmonary artery trunk was found only in 1 patient with tricuspid aortic valve (0/9 vs 1/8, P =.3). During a mean follow-up of 26.5 +/- 12.2 months (32.1 +/- 12.7 vs 20.1 +/- 7.4 months, P =.04), prevalence of pulmonary autograft root dilatation (greater than 4.0 cm) was equally represented in patients with native bicuspid or tricuspid aortic valve (3/9 vs 2/8, P =.6). CONCLUSIONS: Histologic abnormalities of the pulmonary artery root are rare and equally prevalent in young patients with bicuspid and tricuspid aortic valves. On the contrary, root dilatation is relatively common late after autograft root replacement but appears unrelated to bicuspid aortic valve disease or to pre-existing degenerative changes of the pulmonary artery root.     

Selective Sinus Replacement for Aortic Root Aneurysm: Durable Approach in Selected Patients

BackgroundSelective sinus replacement (SSR) allows a tailored repair approach in patients with sinus of Valsalva or asymmetric aortic root aneurysm. SSR avoids the need for coronary reimplantation for nondiseased sinuses and shortens operative time, although potential for late growth of retained sinuses exists. This study describes selection of patients and assesses operative outcomes and late root dimensions after SSR.MethodsFrom 2006 to 2020, 60 patients underwent SSR at a single referral institution. Mixed effect models were used to assess trajectory of postoperative growth of remaining sinuses, adjusting for age of the patient, valve morphology, and baseline root diameter.ResultsMedian age of the patients was 57 (interquartile range [IQR], 48-65) years. Twenty-four (40%) had a bicuspid aortic valve. Most patients (n = 55 [92%]) underwent single sinus replacement (n = 46 noncoronary, n = 9 right), whereas 5 (8%) underwent repair of both the right and noncoronary sinuses. Concomitant aortic valve replacement was performed in 15 patients (25%); aortic valve repair with internal ring annuloplasty or cusp plication was performed in 37 (62%). There was no operative death, stroke, renal failure, or respiratory failure. Median preoperative root diameter was 53 mm (IQR, 51-56 mm) vs 42 mm (IQR, 39-45 mm) at median imaging follow-up of 34 (IQR, 13-49) months. Rate of midterm root growth was 0.2 mm/y, and there were no late root reinterventions.ConclusionsFor patients with sinus of Valsalva or asymmetric root aneurysm, SSR is associated with excellent operative outcomes, and midterm follow-up suggests that the technique is durable. Longer term follow-up is needed to confirm continued stability of the aortic root.     

Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve

BACKGROUND: We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). METHODS: Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. RESULTS: Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). CONCLUSIONS: Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.     

Long-term follow-up of supra-annular pulmonary autograft aortic root replacement in patients with bicuspid aortic valve

Objective: The performance of the Ross procedure in the case of geometric mismatch between pulmonary autograft and a bicuspid aortic root has not yet been fully evaluated. To prevent geometrically caused autograft dysfunction, a modification of the surgical technique is necessary. Methods: Between January 1996 and January 2007, 50 patients (33 male, 17 female; mean age 50+/-14 years; range 13-63 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in 14 cases; insufficiency in 21; combined disease in 15) with a Ross procedure. The pulmonary autograft was inserted partially in supra-annular position to correct the geometric mismatch between the deeper base of the non-coronary sinus and the right/left coronary sinus. In 24 of these patients, additional tailoring of the non-coronary sinus was necessary. In eight patients the non-coronary sinus was covered with a glutaraldehyde treated autologous pericardial patch to prevent pseudoaneurysm formation. Patients were followed up 1, 2, 5 and 10 years postoperatively. Results: There were no early or late deaths. There were six reoperations. One patient was reoperated because of persistent severe aortic valve insufficiency 9 months postoperatively. Three patients were reoperated for formation of subannular pseudoaneurysm, 6, 9 and 30 months postoperatively. One patient was reoperated for closure of a paravalvular dehiscence. Another patient was reoperated 1 year postoperatively because of a severe pulmonary stenosis due to excessive calcification of the bioprosthesis. Echocardiographic follow-up of the remaining patients showed no evidence of residual or recurrent pulmonary autograft regurgitation or progression of aortic root dilatation. Conclusion: Autograft replacement of the bicuspid aortic valve is challenging, as the geometric mismatch has to be adjusted. Valve dysfunction is avoided by a supra-annular implantation technique, but pseudoaneurysm formation at the base of the non-coronary sinus is a worrying aspect. Patch reinforcement may solve this issue.     

Morphometric analysis of aortic media in patients with bicuspid and tricuspid aortic valve

BACKGROUND: Patients with bicuspid aortic valves tend to develop dilatation of the ascending aorta. The aim of this study was to analyze whether or not there is any histologic difference in the aortic media of patients with a bicuspid aortic valve or a tricuspid aortic valve. METHODS: A morphometric analysis of the wall of the ascending aorta was performed in 107 patients with bicuspid aortic valves undergoing aortic valve operations. The thickness of the elastic lamellae of the aortic media and the distances between the elastic lamellae were measured with the use of an image analysis system. The histologic specimens of the ascending aorta from 61 surgical patients with tricuspid aortic valve disease served as a control. RESULTS: The patients with bicuspid aortic valves had thinner elastic lamellae of the aortic media (2.71 +/- 0.23 microm) of the ascending aortic wall than the patients with tricuspid aortic valve disease (2.83 +/- 0.23 microm) (p = 0.006). The patients with bicuspid aortic valves also had greater distances between the elastic lamellae (27.21 +/- 8.69 microm) of the ascending aortic wall in comparison with the patients with tricuspid aortic valve disease (24.34 +/- 5.32 microm) (p = 0.033). There was no difference in the total thickness of the aortic media between the groups (p = 0.62). CONCLUSIONS: Patients with a bicuspid aortic valve had thinner elastic lamellae of the aortic media and greater distances between the elastic lamellae than patients with a tricuspid aortic valve.     

Aneurysm of the noncoronary sinus of valsalva associated with aortic regurgitation: report of a case

A 39-year-old man has complained of palpitation and dyspnea since 8 months ago. With aortic regurgitation pointed out in another hospital, he was referred to our hospital for examination. Preoperative ultracardiography (UCG) showed a dilated noncoronary sinus of Valsalva and aortic regurgitation. A daughter aneurysm was found by aortography. There were 2 perforations and 1 pouch at the noncoronary sinus cusp (NCC). The noncoronary sinus of Valsalva was dilated and the orifice to the daughter aneurysm was noticed. The noncoronary sinus of Valsalva was obliterated by suturing a dacron patch on the orifice. The aortic valve was replaced with ATS 23 mm valve. Postoperative computed tomography (CT) showed clot formation outside the patch.     

Remodeling of the aortic root and reconstruction of the bicuspid aortic valve

BACKGROUND: Currently, isolated reconstruction of a regurgitant bicuspid aortic valve can be performed with adequate early results. Dilatation of the proximal aorta is known to be associated with this valve anomaly and may be partially responsible for the development of primary regurgitation or secondary failure of valve repair. We have used repair of the bicuspid valve with remodeling of the aortic root as an alternative to insertion of a composite graft. METHODS: Between October 1995 and May 1999, 16 patients (12 men, 4 women, aged 35 to 73 years) were seen with a regurgitant bicuspid aortic valve and dilatation of the proximal aorta of more than 50 mm. All patients underwent repair of the valve using either coapting sutures alone (n = 12) or in combination with triangular resection of a median raphe (n = 4). Using a Dacron graft, the aortic root was remodeled and the ascending aorta (n = 16) and proximal arch (n = 4) replaced. RESULTS: No patient died. The postoperative degree of aortic regurgitation was less than grade II in all patients. Valve function has remained stable in all patients between 2 and 43 months postoperatively. CONCLUSIONS: Reconstruction of the regurgitant bicuspid valve in the presence of proximal aortic dilatation is feasible with good results by combining the root remodeling technique with valve repair.     

Post-stenotic aortic dilatation

Aortic stenosis is the most common valvular heart disease affecting up to 4% of the elderly population. It can be associated with dilatation of the ascending aorta and subsequent dissection. Post-stenotic dilatation is seen in patients with AS and/or aortic regurgitation, patients with a haemodynamically normal bicuspid aortic valve and following aortic valve replacement. Controversy exists as to whether to replace the aortic root and ascending aorta at the time of aortic valve replacement, an operation that potentially carries a higher morbidity and mortality.     

Remodeling+Ring(改良Yacoub)技术治疗二叶式主动脉瓣反流合并升主动脉瘤一例

二叶式主动脉瓣患者常合并主动脉扩张,若主动脉瓣质量良好,可行保留主动脉瓣的主动脉根部置换术.本文报道了1例35岁二叶式主动脉瓣反流合并升主动脉瘤男性患者行Remodeling+Ring(改良Yacoub)手术.患者术后第3d复查心脏彩色超声提示主动脉瓣无反流,术后第6d顺利出院.Remodeling+Ring手术保证了...     

Is a bicuspid aortic valve a risk factor for adverse outcome after an autograft procedure?

BACKGROUND: Recently, bicuspid aortic valve disease is posed to be a possible risk factor for dilatation of the pulmonary autograft. METHODS: Analysis of all 123 patients in our prospective cohort with their native aortic valve in situ at the autograft procedure. RESULTS: The bicuspid aortic valve group (n = 81) had more males (p = 0.05), prior cardiac surgery (p = 0.02), prior aortic valve balloon dilatation (p = 0.01), aortic stenosis (p = 0.03), and less deterioration of left ventricular function (p = 0.02) than the tricuspid group (n = 42). Hospital mortality occurred in 3 patients (bicuspid 2, tricuspid 1). The follow-up was 99% complete (median, 5.3 years; SD, 3.5; range, 0.1 to 13.4) with a total of 674 patient years. During follow-up 4 patients died (bicuspid 2, tricuspid 2). Overall survival was 95% (95% confidence interval [CI], 89% to 98%) at 5 and 10 years. Seven patients required reoperation for autograft failure, all structural. Freedom from autograft reintervention was 97% (95% CI, 92% to 100%) at 5 years and 89% (95% CI, 79% to 98%) at 10 years. There were no differences in outcome between the groups. Four patients required reoperation for allograft failure, all structural. Freedom from allograft reoperation was 99% (95% CI, 97% to 100%) at 5 years and 91% (95% CI, 82% to 100%) at 10 years. There was no difference between the groups. CONCLUSIONS: An autograft procedure in patients with a bicuspid aortic valve is justified. Bicuspid aortic valve disease is not a contraindication for an autograft procedure. Patients with a bicuspid aortic valve will meet the limitations of the autograft procedure in the same frequency as the overall autograft population.     

Absence of right superior vena cava and aortic annular hypoplasia in a patient with Turner's syndrome

Cardiovascular abnormalities are frequently encountered in patients with Turner's syndrome. These include coarctation of the aorta, bicuspid aortic valve, aortic root dilatation, atrial and ventricular septal defects, but absence of the right superior vena cava (SVC) in visceroatrial situs solitus is extremely rare. We report absence of the right SVC and congenital aortic annular hypoplasia with bicuspid aortic valve stenosis in a patient with Turner's syndrome. Aortic root extension with prosthetic valve replacement was performed and absent right SVC, which was detected incidentally during operation, is confirmed by postoperative venous angiogram.     

Austrian triad with sinus of Valsalva aneurysm and rupture

An interesting case of a young patient presenting with Austrian's Triad: pneumococcal pneumonia, pneumococcal meningitis, and pneumococcal endocarditis. On echocardiogram the patient was noted to have a noncoronary sinus of Valsalva aneurysm and vegetations on the aortic cusps resulting in disruption of valvular integrity and severe aortic insufficiency. Color Doppler also revealed rupture of the aneurysm into the right atrium. The patient was taken to surgery where the noncoronary cusp was noted to be completely replaced with vegetative lesions. The aortic valve was replaced with a No. 21 Carpentier-Edwards bioprosthetic valve (Edwards Lifesciences, Irvine, CA), and the noncoronary sinus of Valsalva was repaired with concomitant closure of the fistula using glutaraldehyde autologous pericardium.     

A case report of aortic valve replacement following ruptured aneurysm of the sinus of valsalva with bicuspid valve

Only three cases of the combination of bicuspid aortic valve and ruptured aneurysm of the sinus of Valsalva, associated with previously repaired coarctation of aorta, have been reported. A twenty-year-old man with a sudden onset of CHF due to ruptured aneurysm of the sinus of Valsalva underwent intracardiac repair by direct closure of the sinus Valsalva in combination with patch closure of a subarterial VSD. Although, no AR was detected preoperatively, massive reguratitation occurred after the repair due to subsequent failure of aortic valve coaptation in the present of the bicuspid aortic valve, which was not diagnosed preoperatively. Aortic valve replacement with SJM 25 mm was successfully performed.